Emma Coto and her family met with doctor after doctor looking for answers. They found them at Boston Children’s Hospital.

CLEARWATER — It’s easier to feel than to see the slight bump on Emma Coto’s head.

It comes from a small electrical device, reminiscent of the bottom half of an old flip phone, that doctors implanted below her scalp. About 250 times a day, it sends a tiny electrical charge through two wires directly into her brain.

The medical treatment for epilepsy known as responsive neurostimulation, or RNS, was a long shot for Emma. The U.S. Food and Drug Administration has approved it only for adults, and it was doubtful whether insurance would pay for this expensive, unauthorized procedure.

But after years of fighting for the best treatment for their daughter, parents Lynda and Danny Coto weren’t ready to give up on a possible answer to their prayers.

Emma was just 72 hours old when she experienced an episode of respiratory arrest, which likely caused her epilepsy. The Clearwater couple didn’t hesitate to transfer Emma to different hospitals, including a Learjet trip to Philadelphia, when doctors ran out of answers.

For the next 7 years, the couple refused to accept the opinion of neurologists who said their daughter would be developmentally delayed and that, even with medication, she would routinely suffer seizures during which she’d stop breathing.

Their journey led them to Boston Children’s Hospital in 2020, where Emma became the hospital’s youngest RNS recipient.

Now 11, Emma reads at grade level at her Safety Harbor Montessori school. She plays basketball and flag football and gives piano recitals. It’s been almost three years since her last clinical seizure.

“It seemed like a goal that maybe we could reach in five years, if she were still alive,” Lynda said. “For everything to fall in place the way that it did was nothing short of divine intervention.”

Challenging doctors

Lynda and Danny Coto both hail from New Orleans but they first met at MacDintons in Tampa’s Soho neighborhood.

They had been previously married and already were parents but they wanted their own child after they wed in 2007.

Emma was born in 2012. Just three hours after mom and daughter were discharged from the hospital, Emma stopped breathing and turned blue.

Without intervention within about five minutes, respiratory arrest can irreversibly damage organs, including the brain. Lynda, who at the time was a registered nurse, performed CPR while Emma’s grandmother called 911.

At the local emergency room, doctors discovered Emma had an alarmingly low level of glucose, the main energy source for the brain.

Doctors stabilized her with an intravenous drip but had no answer for why it happened. Emma was hospitalized for more than a month, during which time her parents moved her to a different hospital every time they felt doctors had exhausted their expertise.

It wasn’t until she went to the Children’s Hospital of Philadelphia on a specially adapted Learjet operated by nonprofit group Angel Flight that doctors discovered that her pancreas was riddled with benign tumors.

The infant underwent surgery to remove the tumors leaving her with about 15% of her pancreas. It stabilized her, but the glucose deficiency had left her prone to seizures.

The Centers for Disease Control and Prevention estimate that 1.2% of people in the United States have active epilepsy. Its chief symptom is seizure, caused by sudden and uncoordinated changes to electrical signals in the brain.

In Emma’s case, those signals come from the occipital lobes, two areas at the back of the brain that control vision. Emma was put on anti-seizure medication but by the time she was 2, her mom would catch her spacing out for periods or repeatedly blinking and being unresponsive.

Brain scans eventually showed that those episodes were seizures. Over the next three years, her seizures became more frequent, long-lasting and intense. At times, she would stop breathing until given rescue medication, leaving her parents fearing any such episode could end fatally.

Her parents were told the damage to the occipital lobes would mean she had cerebral visual impairment, a disorder caused by damage to the parts of the brain that process vision.

She may not recognize you, one doctor told Lynda. She will see things but never will be able to tell you what they are. Emma walked with a cane for a while and wore glasses but still bumped into objects.

Doctors advised anti-seizure medication as a way to reduce the frequency of seizures but there seemed to be no good outcomes. Because her seizures originated in a part of the brain that controls vision, surgery to remove the parts of her brain that triggered seizures could have left her blind.

Lynda describes herself as a rule follower. But when it came to her daughter, her medical training gave her the confidence to challenge doctors. After one specialist recommended a medically induced coma to calm her brain, she lost her patience.

“I told him, ‘I don’t just expect my daughter to live, I expect my daughter to have a quality of life,’” Lynda said. “‘I want her to go to school. I want her to wake up in the morning and not have to worry about seizures. I want her to be able to go out on a field and play soccer or play softball.’”

Finding an answer

Lynda stumbled upon RNS while researching epilepsy treatments on the internet. Seizures in patients undergoing the medical treatment typically decrease by about 50% within two years, according to the Epilepsy Foundation.

The treatment was offered at Boston Children’s, which has been ranked No. 1 or No. 2 in the U.S News & World Report’s Best Children’s Hospitals list 13 years in a row.

The hospital was within a two-hour drive of the Cotos’ vacation home in Provincetown. Lynda was able to get a consultation with an epileptologist through the hospital’s Second Opinion Program. She calls it the lifesaver line.

The Cotos traveled to Boston in May 2019 and were told Emma was a good candidate for RSN despite her age.

“For the first time in years I had a doctor, look at me and say, ‘Everything you’re saying makes complete sense, and I think we can help you,’” Lynda said.

To get the expensive procedure covered through insurance, the Cotos had to convince their provider that they had exhausted every other option.

Before the surgery, neurologists had to wean her off anti-seizure medication and record seizure activity using an electroencephalogram, known as an EEG, to determine the exact brain locations where her seizures were starting.

During surgery, her doctors carved out a small niche on top of her skull to make the device flush to her head. It contains a central processing unit and battery that will last about 8 years. It has two wires threaded through small holes in the skull that connect to the surface of the brain where her seizures originate.

For the first few months, neurologist Melissa Tsuboyama used the device to record brain activity, allowing her to see the pattern of brain waves before and during seizures.

That enabled her to program the device, which can be done remotely, setting it to recognize early seizure activity and to head it off by sending small bursts of electrical stimulation that help return brain waves to normal. The electrical charge is so minor that Emma is unaware when it fires.

“We know now that the brain seems to like having this constant or frequent electrical stimulation and it helps essentially reorganize its networks,” Tsuboyama said. “A brain that is perhaps originally programmed to have more seizures (can) retrain itself to not have those seizures.”

Being seizure-free has allowed Emma to live a near normal life. She is a fan of Taylor Swift and Billie Eilish, but her musical tastes also include conductor John Williams, especially for his musical score for Jurassic Park, a movie that inspired her love of dinosaurs.

“I want to be a singer and a paleontologist,” she said when asked about a career.

While Emma has not had a clinical seizure since May 2020, her brain still has damaged tissue and she is still being monitored.

Once a day her mom downloads her brain wave activity from the device by holding an electronic wand connected to a laptop against Emma’s skull. A screen on the laptop records the data transfer and when it’s complete the information is sent electronically to Tsuboyama.

Companies that manufacture RNS have been trying to get the federal government to lower the approval age to make it easier for parents to pay for the treatment, Tsuboyama said. A neurostimulator can cost as much as $50,000 while the treatment also requires consultation, surgery and aftercare and ongoing monitoring.

It’s not lost on Tsuboyama that Lynda’s familiarity with the health care system gave her the tools to advocate for her daughter. She’d like this type of treatment to be an option for every patient.

“It is a big struggle to not be able to have this available to other people who might be excellent candidates,” she said.

 

Source: tampabay.com, Christopher O’Donnell

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